Behçet Disease

Common Symptoms


Ocular complaints (noted in up to 50% of Behçet patients) are usually bilateral; symptoms include photophobia, eye pain, decreased vision, red eye, and floaters. Systemic manifestations, including oral and genital ulcers, usually precede ocular manifestations (▶ Table 70.1).












Table 70.1 Behçet’s Disease Research Committee of Japan diagnostic criteria (92% sensitivity and 89% specificity). All four major criteria are required for the diagnosis of complete-type Behçet disease. An incomplete-type diagnosis consists of the presence of three major criteria, two major and two minor criteria, ocular disease plus one major criterion, or ocular disease plus two minor criteria. (Data from Lehner et al. 1979; Behçet’s Disease Research Committee of Japan, 1987.)

Major criteria




  • Recurrent oral aphthous ulcers (nearly all patients; must have three recurrences over 12-mo period)



  • Skin lesions (seen in up to 80%; erythema nodosum-like lesions, folliculitis, acneiform lesions, thrombophlebitis, and cutaneous hypersensitivity)



  • Genital ulcers



  • Ocular disease (iridocyclitis and posterior uveitis)

Minor criteria




  • Arthritis (50%; affects knees, ankles, wrists, and elbows)



  • Epididymitis



  • Intestinal symptoms attributed to ileocecal ulcerations



  • Vascular symptoms



  • Neurologic symptoms (headache, cranial nerve palsies, seizures, stroke, meningitis, etc.)


Source: Adapted from Lehner et al. 1979; Behçet’s Disease Research Committee of Japan, 1987.


70.1.2 Exam Findings


Patients develop recurrent, nongranulomatous uveitis (anterior, intermediate, posterior, or most commonly panuveitis). Anterior segment findings include cell, flare, keratic precipitates, cyclitic membranes, posterior synechiae, and/or anterior synechiae. Other possible anterior segment findings include hypopyon (12%) with or without conjunctival injection; cataracts (cortical secondary to inflammation and posterior subcapsular secondary to corticosteroid treatment); episcleritis or ciliary flush; neovascularization of the iris; seclusio pupillae (with the associated risk of secondary pupillary block glaucoma); and uncommonly conjunctival ulcer. Posterior segment findings vary (▶ Table 70.2) but often include vitritis, retinal vasculitis, retinal hemorrhage, and areas of retinitis (▶ Fig. 70.1 ▶ Fig. 70.2). Phthisis bulbi may be observed in end-stage disease.
















Table 70.2 Posterior segment findings in Behçet disease

Vitreous


  • Vitreous cell or haze



  • Vitreous hemorrhage



  • Vitreous snowbanking/snowballs


Optic nerve


  • Disc edema



  • Neovascularization of the disc (▶ Fig. 70.1)



  • Pallor



  • Disc hyperemia


Retina


  • Retinal vasculitis (affecting arterioles and venules)



  • Retinitis



  • Cystoid macular edema



  • Intraretinal hemorrhage



  • Neovascularization



  • Chorioretinitis



  • Epiretinal membrane



  • Macular hole



  • Retinal vascular occlusion (secondary to obliterative vasculitis)



  • Exudative or tractional retinal detachment



  • Retinal pigment epithelial atrophy



(a) Ultra-widefield fundus photograph and (b) optical coherence tomography (OCT) of active Behçet disease. The fundus photograph demonstrates multiple cotton wool spots from an active flare. The corre


Fig. 70.1 (a) Ultra-widefield fundus photograph and (b) optical coherence tomography (OCT) of active Behçet disease. The fundus photograph demonstrates multiple cotton wool spots from an active flare. The corresponding OCT on the right shows inner retinal hyperreflectivity and retinal thickening consistent with retinal ischemia and ongoing inflammation.

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Mar 24, 2020 | Posted by in OPHTHALMOLOGY | Comments Off on Behçet Disease

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